Leiomyosarcoma of the uterus with sphenoid bone and orbital metastases

A 55-year-old woman presented with a 1-week history of vision loss in the right eye associated with proptosis and diplopia. Past medical history was significant for high-grade leiomyosarcoma of the uterus status post total abdominal hysterectomy and bilateral salpingo-oophorectomy and postoperative pelvic radiation 18 months prior to presentation. Staging studies at the time of initial diagnosis of uterine leiomyosarcoma showed no evidence for metastatic disease. At presentation, CT and MRI showed a well-circumscribed 3.0 cm x 3.6 cm x 2.4 cm mass centered in the right greater sphenoid wing, extending into the middle cranial fossa and the superior and lateral orbital wall. Biopsy of the orbital mass revealed a poorly differentiated high-grade leiomyosarcoma, consistent with recurrent metastatic disease from the uterus. The patient subsequently underwent radiation treatment followed by a left orbital exenteration 6 months after the orbital biopsy. A left thoracostomy was performed 8 months after the orbital biopsy for a metastatic nodule in the left lower lobe of the lung. The clinicopathologic findings of this rare metastatic orbital lesion are presented.